Although mutations in <i>CYP11B1</i> that cause steroid 11β-hydroxylase deficiency more commonly lead to the CAH phenotype, cases have been reported of <i>CYP11B1</i> mutations leading to NCCAH, depending on the location of the mutations.<h4>Conclusion</h4>This patient's case demonstrates physical examination and laboratory findings suggestive of NCCAH. Here, CYP11B1 is linked to congenital adrenal hyperplasia.